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Sialic acid storage disease.

Authors :
Cameron, P. D.
Dubowitz, V.
Besley, G. T. N
Fensom, A. H.
Besley, G T
Source :
Archives of Disease in Childhood; Mar1990, Vol. 65 Issue 3, p314-315, 2p
Publication Year :
1990

Abstract

A baby girl with coarse facial features, hepatosplenomegaly, and developmental delay had raised free sialic acid concentrations in her urine and cultured fibroblasts. She died aged 13 months. Sialic acid is an important constituent of many glycoproteins and glycolipids; impaired release from the lysosome may be the underlying biochemical defect. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00039888
Volume :
65
Issue :
3
Database :
Complementary Index
Journal :
Archives of Disease in Childhood
Publication Type :
Academic Journal
Accession number :
66707163
Full Text :
https://doi.org/10.1136/adc.65.3.314